Topic Contents

Castleman's Disease

National Organization for Rare Disorders, Inc.

ImportantIt is possible that the main title of the report Castleman's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

angiofollicular lymph node hyperplasia

angiomatous lymphoid

giant benign lymphoma

giant lymph node hyperplasia

hamartoma of the lymphatics

Castleman tumor

Disorder Subdivisions

None

General Discussion

Castleman's disease is a rare disorder characterized by non-cancerous (benign) growths (tumors) that may develop in the lymph node tissue throughout the body (i.e., systemic disease). Most often, they occur in the chest, abdomen, and/or neck, but may also be found in the armpit (axilla), pelvis, and pancreas. Usually the growths represent abnormal enlargement of the lymph nodes normally found in these areas.

Castleman's disease may be divided into four types. There are two main types of Castleman's disease that are determined by the microscopic appearance of the lymph node: hyaline-vascular type and plasma cell type. The hyaline vascular type accounts for approximately 90 percent of the cases. Most individuals exhibit no symptoms of this form of the disorder (asymptomatic) or they may develop non-cancerous growths in the lymph nodes. The plasma cell type of Castleman's disease may be associated with fever, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/or abnormally increased amounts of certain immune factors in the blood (hypergammaglobulinemia). Additionally, Castleman's disease can be divided into categories which are defined by number of anatomic body regions affected by the disease. Unicentric Castleman's disease affects lymph nodes in only one part of the body, while multicentric Castleman's disease affects multiple parts of the body. A person can have any combination of the microscopic and anatomic variants, thus there are four possibilities: unicentric hyaline vascular, unicentric plasma cell variant, multicentric hyaline vascular variant, and multicentric plasma cell variant. Many individuals with multicentric Castleman's disease may exhibit an abnormally large liver and spleen (hepatosplenomegaly).

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

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